Second international guidelines for the diagnosis and management of hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications. The guidelines were developed using the AGREE II (Appraisal of Guidelines for Research and Evaluation II) framework and GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. The guidelines expert panel included expert physicians (clinical and genetic) in HHT from 15 countries, guidelines methodologists, health care workers, health care administrators, patient advocacy representatives, and persons with HHT. During the preconference process, the expert panel generated clinically relevant questions in 6 priority topic areas. A systematic literature search was done in June 2019, and articles meeting a priori criteria were included to generate evidence tables, which were used as the basis for recommendation development. The expert panel subsequently convened during a guidelines conference to conduct a structured consensus process, during which recommendations reaching at least 80% consensus were discussed and approved. The expert panel generated and approved 6 new recommendations for each of the following 6 priority topic areas: epistaxis, gastrointestinal bleeding, anemia and iron deficiency, liver VMs, pediatric care, and pregnancy and delivery (36 total). The recommendations highlight new evidence in existing topics from the first International HHT Guidelines and provide guidance in 3 new areas: anemia, pediatrics, and pregnancy and delivery. These recommendations should facilitate implementation of key components of HHT care into clinical practice.

Radiologic peroral gastrostomy.

PURPOSE: To develop an improved percutaneous technique for the insertion of large-bore gastrostomy tubes. MATERIALS AND METHODS: With use of fluoroscopic guidance, the stomach is punctured and the esophagus is catheterized in a retrograde fashion. A guide wire is passed from the gastrostomy site, up the esophagus, and out of the patient's mouth. A large-bore (20-24 F) endoscopic push-type gastrostomy tube is advanced over the wire, through the mouth, down the esophagus, and out of the gastrostomy site. RESULTS: Thirty-one successful tube placements were performed in 32 attempts (97% success rate). There were no major procedural or postprocedure complications. Minor complications included one lip laceration (one of 31 = 3%), one minor exit site infection (one of 31 = 3%), and two inadvertent tube dislodgements (two of 31 = 6%). CONCLUSIONS: Radiologic placement of large-bore endoscopic gastrostomy tubes is possible without endoscopy. The procedure is rapid, easy to perform, and safe.

Lymphokine-induced production and release of lysosomal enzymes by macrophages.

MACROPHAGES ARE ASSOCIATED WITH MOST CHRONIC INFLAMMATORY LESIONS, AND THESE CELLS CONTAIN ENZYMES THAT ARE ABLE TO DESTROY CONNECTIVE TISSUE CONSTITUENTS. Normal lymphoid cells responding to a mitogen, phytohemagglutinin-P, release factor(s) that cause a marked increase in the size and enzyme content for mononuclear phagocytes maintained in culture. The stimulated macrophages, which by several criteria remain otherwise viable and healthy, selectively release large quantities of hydrolytic enzymes to the culture medium.